On harelip and cleft palate

by William Rose

Harelip.

French, bec-de-lièvre. German, Hasenscharte—or if with complete cleft palate, Wolfsrachen (wolf-jaw).

Fig. 1.—Hare’s lip to show the median cleft in the lower part prolonged upwards into either nostril. (Sutton.)

Harelip is a congenital deformity of the upper lip, characterised by a cleft extending for a variable depth, either through the soft tissues of the lip only, or implicating in addition the alveolus, floor of the nose, and palate. No mention of this condition is made by Hippocrates, Galen, or any of the fathers of medicine; and so far as I can discover the name is first used by Ambrose Paré, who probably initiated the treatment by pin and figure-of-8 suture. The name is really a misnomer, in that the condition (as has been many times pointed out, but notably by Fergusson) does not simulate a hare’s lip except in the fact of being cleft, for the natural cleft in the animal’s lip is always in the median line below, bifurcating above to reach either nostril ([Fig. 1]), whereas in the abnormal human lip the cleft lies to one or the other side. Instances of median defect are known, but they are extremely uncommon, and consist often of more than a simple fissure.

Fig. 2. Fig. 3. Fig. 4.

Fig. 5. Fig. 6.

The deformity may exist as a simple notch in the soft tissues of the lip, unilateral ([Figs. 2 and 3]) or bilateral ([Fig. 4]); when more decided, it may implicate one or both nostrils ([Figs. 5, 6, and 7]). In mild cases the alveolus is intact; in others, cleft, constituting the variety known as alveolar harelip, and the line of fissure may, or may not, extend backwards into the palate. In all cases of double alveolar cleft, the palate is also involved, and the central parts of the lip and intermaxilla tend to project forwards; in the severest forms these portions are completely isolated from the maxillæ, and, supported by the vomer and septum nasi, form a proboscis-like appendage to the end of the nose, which is excessively disfiguring. ([Figs. 7 and 8] illustrate this deformity as seen from the front and in profile.)

The shape of the nose in unilateral harelip is very characteristic, being broad and flattened out from the deficiency of the floor and posterior wall of the anterior nares.

Figs. 7 and 8.—Double harelip with projection of the os incisivum, as seen from the front and in profile. (Fergusson.)

Harelip seems to occur more commonly in boys than in girls. According to Müller, out of 270 cases, 170 were boys, and 100 girls.

Unilateral harelip is more commonly met with on the left side than on the right; probably 60-70 per cent. of the cases are left-sided. Thus Müller reports 142 left-sided against 62 right-sided clefts; Mason, out of 65 cases, found 54 to be unilateral, and of these 35 left-sided to 19 on the right; Kölliker mentions that in 165 unilateral clefts, 113 were on the left side, and 62 on the right. My own experience quite coincides with these figures. At present, no satisfactory explanation of this preponderance of left-sided clefts has been given. One solution suggests itself, but we have no facts of importance to support it, viz. that, inasmuch as the majority of people are from heredity or education right-handed, Nature devotes more energy to completing her developmental processes on that side than on the left, and any check to this would be more likely to happen on the left side. It would be valuable and interesting to know in what proportions other unilateral deformities occur on the left and right sides respectively.

Occasionally one sees in the upper lips of children a congenital red line apparently cicatricial, occupying the position of the normal harelip fissure, and which has been supposed to indicate a natural cure of a temporary defect of development. My colleague, Mr. Carless, has recently shown me a case of this character under his care in a child a few weeks old. There was a well-marked red line extending from the lip margin to the nostril; but there was no irregularity in the red border, and no evidence of cicatricial contraction; the tissue of the lip, moreover, seemed quite soft and normal, not fibrous or hard. These points seem to bear out fully Trendelenburg’s opinion[1] that the name “intra-uterine cicatrisation or cure of a harelip” is incorrect, and that such cases are simply due to the raphe of union remaining evident instead of disappearing as usual; and he quotes the normal appearance of the raphes in the scrotum and perinæum as similar conditions. In this child there was no evidence of any groove or depression in the alveolus; but other deformities were present, viz. a very definite post-anal dimple, the cicatrix being adherent to the tip of the coccyx, a slight condition of hypospadias, and a congenital hydrocele. There was no history of deformity in the family, nor of maternal impression.